The impact of interruption of anomalous systemic arterial supply on scimitar syndrome presenting during infancy

Uthaman, Babu; Abushaban, Lulu; Al-Qbandi, Mustafa; Rathinasamy, Jebaraj

doi:10.1002/ccd.21430

Cited by 33 publications

(47 citation statements)

References 14 publications

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“…Interestingly, coil embolisation or surgical ligation of systemic arterial collateral has been performed for management of pulmonary artery hypertension in children with a similar disorder, i.e. Scimitar syndrome [10].…”

Section: Discussionmentioning

confidence: 99%

Asymptomatic adults with isolated, unilateral right pulmonary vein atresia: multidetector CT findings

Kim¹,

Yoo²,

Ahn³

et al. 2011

BJR

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ABSTRACT. We report two cases of a very rare congenital anomaly, i.e. isolated unilateral pulmonary vein atresia. The patients were asymptomatic and the diagnosis was made using multidetector CT (MDCT), which also showed cyst formation in the right lung. Asymptomatic adult cases or association with cystic lung lesions have never been reported in this condition before. Unilateral pulmonary vein atresia without associated congenital heart disease is a rare condition [1]. Patients with congenital unilateral pulmonary vein atresia are usually symptomatic and present with recurrent episodes of pneumonia or haemoptysis in infancy or childhood [1]. Although a subclinical course of this anomaly has been recently reported in a 12-year-old boy, so far no asymptomatic adult cases have been described to our knowledge [1]. Also, the presence of pulmonary cysts has never been reported in this condition. We therefore report the CT findings of two asymptomatic adult patients with unilateral pulmonary vein atresia, with associated pulmonary cysts. Case report Case 1A previously healthy 23-year-old male visited the emergency room because of vomiting lasting 1 day. The chest radiograph obtained at the time showed diffuse interstitial infiltration in the right lung field. Multidetector CT (MDCT) showed a heterogeneous lung density, with areas of normal density, ground-glass opacity and small air-cysts (Figure 1a). The cysts were multiple, but predominantly distributed in the subpleural regions. Beaded thickening of the interlobular septum and nodularity along the fissures and costal pleura were noted in the right lower lung (Figure 1b). The volume of the right lung was not diminished. CT also showed a complete lack of the right pulmonary vein and enlargement of the left. The margin of the left atrium where the right pulmonary vein was expected to be was entirely smooth, with no vascular structures connected to it (Figure 1c). The right pulmonary artery was slightly small, with poor contrast enhancement compared with the left pulmonary artery. The mediastinal branches of the right bronchial artery were markedly dilated, suggesting the presence of systemic arterial flow into the hypoplastic pulmonary arteries (Figure 1d). Anomalous pulmonary venous return was excluded on the basis of the absence of pulmonary venous structures connected to systemic venous circulation on CT.Fibre-optic endoscopy revealed findings compatible with reflux oesophagitis and chronic superficial gastritis. The patient recalled being told that his chest radiography showed a ''small right pulmonary artery''. He did not complain of any respiratory, cardiac or other systemic symptoms, and refused further work-up. The patient was in good health 5 years after the initial emergency room visit. Case 2A 39-year-old female with rheumatoid arthritis recently underwent screening chest radiography in preparation for a clinical trial (Figure 2a). This showed a small right hemithorax with significant ipsilateral mediastinal shift, reduced vessel size in the hilar region and...

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Section: Discussionmentioning

confidence: 99%

Asymptomatic adults with isolated, unilateral right pulmonary vein atresia: multidetector CT findings

Kim¹,

Yoo²,

Ahn³

et al. 2011

BJR

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“…2,4,5,7,10,15,20 Because of the wide range of symptoms at clinical onset, the diagnosis may be difficult, especially in children and young adults with concomitant congenital heart lesions. [2][3][4][5]32,33 Pulmonary artery hypertension often occurs and is seen mostly in infants 12,14,16 -18,20 with associated congenital heart malformations or with an anomalous large systemic arterial supply.…”

Section: Discussionmentioning

confidence: 99%

“…38 A less common cause of pulmonary arterial hypertension is the presence of pulmonary vein stenosis. 16,17,20 If the presence of a large systemic anomalous arterial supply to the right lung with pulmonary overcirculation is detected preoperatively, it should be abolished by occluding the systemic arteries that supply the affected lobe(s), thereby reducing the blood flow and the amount of shunting. 15,16,20,[32][33][34][37][38][39][40] This has been advocated as the best and simplest treatment, especially in infants with congestive heart failure, often improving the clinical symptoms and decreasing the pulmonary arterial pressure.…”

Section: Discussionmentioning

confidence: 99%

“…16,17,20 If the presence of a large systemic anomalous arterial supply to the right lung with pulmonary overcirculation is detected preoperatively, it should be abolished by occluding the systemic arteries that supply the affected lobe(s), thereby reducing the blood flow and the amount of shunting. 15,16,20,[32][33][34][37][38][39][40] This has been advocated as the best and simplest treatment, especially in infants with congestive heart failure, often improving the clinical symptoms and decreasing the pulmonary arterial pressure. 1,14,[17][18][19] The surgical repair of scimitar syndrome consists of redirecting the pulmonary venous drainage into the left atrium, 14,15 either baffling the anomalous drainage into the left atrium via a tunnel or transecting the "scimitar drainage" near its entrance into the inferior vena cava and then reimplanting it directly into the left atrium.…”

Section: Discussionmentioning

confidence: 99%

“…16 Overall, 19% to 31% of patients with scimitar syndrome have associated cardiac anomalies. 1,10,11,15,20 This syndrome can present early in the neonatal period or later in life with a wide clinical spectrum. 4,14,16,21 Dupuis et al 19 divided scimitar syndrome into 3 main forms: an infantile form with symptoms and pulmonary hypertension, an "older" adult form distinguished by being asymptomatic in infancy, and a form with associated congenital cardiac anomalies.…”

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Scimitar Syndrome

Vida¹,

Padalino²,

Boccuzzo³

et al. 2010

Circulation

140

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Background— Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. Methods and Results— From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. Conclusions— The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.

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Scimitar Syndrome

2009

The Natural and Unnatural History of Congenital Heart Disease

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The impact of interruption of anomalous systemic arterial supply on scimitar syndrome presenting during infancy

Abstract: Interruption of ASAS helps to avoid or defer definitive surgery for SS during infancy. Therefore, we recommend coil embolization of ASAS as initial palliation, and long term surveillance to assess need for further intervention.

Cited by 33 publications

References 14 publications

Asymptomatic adults with isolated, unilateral right pulmonary vein atresia: multidetector CT findings

Asymptomatic adults with isolated, unilateral right pulmonary vein atresia: multidetector CT findings

Scimitar Syndrome

Scimitar Syndrome

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